Catastrophic antiphospholipid syndrome presenting as bilateral central retinal artery occlusions

Abstract

A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial "boxcarring," retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient's vision improved and remained stable at 20/200 and 20/80.

Figure 1

Color fundus photos of each eye show bilateral retinal arterial “boxcarring,” retinal whitening, intraretinal and preretinal hemorrhages, and cherry red spots.

Figure 2

Fluorescein angiography of both eyes in the late phases shows minimal perfusion to the peripapillary areas with late leakage of the filling vessels.

Figure 3

Optical coherence tomography of maculae shows cystoid macular edema and loss of foveal contour.